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Degos Disease
Abstract
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NORD is very grateful to Rachel Vale, BSc, University of Ottawa and Noah Scheinfeld, MD, Assistant Clinical Professor of Dermatology, Columbia University, for assistance in the preparation of this report.
Synonyms of Degos Disease
- Degos-Kohlmeier disease
- Degos syndrome
- Kohlmeier-Degos disease
- malignant atrophic papulosis
- MAP
- papulosis atrophicans maligna
Disorder Subdivisions
- No subdivisions found.
General Discussion
Degos disease is an extremely rare disorder in which small and medium sized arteries become blocked (occlusive arteriopathy), restricting the flow of blood to affected areas. Degos disease usually causes characteristic skin lesions that may last for a period of time ranging from weeks to years. In some individuals, Degos disease will be limited to the skin (benign cutaneous Degos disease); other individuals will also develop symptoms affecting other organ systems (systemic Degos disease). Systemic Degos disease is most frequently characterized by lesions in the small intestine, but other organs are also affected. Major symptoms may include abdominal pain, diarrhea, and/or weight loss. The systemic form of Degos disease can cause life-threatening complications such as perforated bowels leading to inflammation of the abdominal cavity (peritonitis). The exact cause of Degos disease is unknown.
Many researchers caution that statistics concerning Degos disease are inaccurate because many individuals go undiagnosed and most medical reports disproportionately discuss the more serious systemic form. It is important to note that some individuals only develop skin lesions (which are not associated with life-threatening complications) and do not go on to develop systemic Degos disease. Affected individuals should talk to their physicians and medical team about their specific case and associated symptoms.
Organizations related to Degos Disease
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